[Hearing loss leading to the diagnosis of granulomatosis with polyangiitis: An unusual case in the Afro-Caribbean population].

Abstract

Granulomatosis with polyangiitis (GPA) was diagnosed in a 65-year-old Afro-Caribbean patient presenting initially with hearing loss and a pseudo-tumoral 6cm lung mass. Lung biopsy findings favored the diagnosis of vasculitis. Rapid disease progression was noted with near-complete deafness and lack of speech, severe renal failure necessitating dialysis, and persisting disturbance of consciousness following tonic-clonic seizures due to posterior reversible encephalopathy syndrome (PRES). The patient died one month after admission due to ARDS secondary to ventilator-associated pneumonia. Otological symptoms are frequently the first signs of GPA and should alert the clinician when concomitant with lung nodules, even among Afro-Caribbean patients, in whom GPA is unusual. GPA is a rare disease occurring nearly exclusively in Caucasian populations and is associated with anti-neutrophil cytoplasm antibodies (ANCA) with anti-proteinase 3 (PR3) specificity. Diagnosis is based on clinical, radiological, and biological findings. While pathology from lung localizations is inconsistently specific and rarely made, it can help to establish the diagnosis. This clinical case aptly illustrates the specific clinical presentation of GPA and the potential severity of its multi-organ manifestations.